Wernicke's Encephalopathy and Korsakoff's Syndrome

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: closed (20 September 2023) | Viewed by 39838

Special Issue Editors


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Guest Editor
Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands
Interests: cognitive and clinical neuropsychology; neuropsychiatry

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Guest Editor
Department of Medicine for Older People, Amsterdam Public Health Research Institute, Amsterdam University Medical Centres, Location VUmc, Amsterdam, The Netherlands
Interests: medical care in advanced disease; geriatric palliative care; care ethics

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Guest Editor
Lelie Care Group, Slingedael Korsakoff Center, Rotterdam, The Netherlands
Interests: nonpharmacological treatment; long-term care; neuropsychology; neuropsychiatry

Special Issue Information

Dear Colleagues,

Wernicke’s encephalopathy and Korsakoff’s syndrome are neuropsychiatric syndromes that may occur in the context of malnutrition and thiamine deficiency, often—but not exclusively—in the context of alcohol use disorder. Despite the fact that thiamine supplementation in individuals at risk for developing Korsakoff’syndrome is available, there are still many patients that are newly diagnosed worldwide, alhough exact numbers are lacking. In addition, many people who have been diagnosed with Korsakoff’s syndrome still have to cope with the long-term consequences of this disorder that affect the everyday life of these individuals. In this Special Issue, we welcome authors to submit papers on Wernicke’s encephalopathy and/or Korsakoff’s syndrome. Topics may include—but are not limited to—their assessment and diagnostic work-up, their neuropsychiatric or cognitive sequelae, the neuropathology of these syndromes, somatic comorbidity, pharmacological and nonpharmacological treatments, quality of life, mental capacity, patient care and nursing, epidemiology, or neuroimaging research. Note that we welcome submissions on both alcoholic and nonalcoholic Wernicke’s encephalopathy and Korsakoff’s syndrome.

Prof. Dr. Roy P.C. Kessels
Prof. Dr. Cees M.P.M. Hertogh
Dr. Erik Oudman
Guest Editors

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Keywords

  • alcohol-related cognitive disorders
  • neuropsychiatry
  • neuropsychology
  • thiamine
  • addiction
  • nursing

Published Papers (19 papers)

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Research

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14 pages, 273 KiB  
Article
Over a Century of Study and Still Misunderstood: Recognizing the Spectrum of Acute and Chronic Wernicke–Korsakoff Syndrome
by Simon J. Scalzo and Stephen C. Bowden
J. Clin. Med. 2023, 12(21), 6880; https://doi.org/10.3390/jcm12216880 - 31 Oct 2023
Viewed by 1287
Abstract
The aim of this study was to objectively evaluate the hypothesis that the neuropsychological presentation of Korsakoff’s syndrome, the chronic phase of Wernicke–Korsakoff syndrome (WKS), is invariably a severe, selective amnesia against a background of relatively preserved general intellectual functions in a consecutive [...] Read more.
The aim of this study was to objectively evaluate the hypothesis that the neuropsychological presentation of Korsakoff’s syndrome, the chronic phase of Wernicke–Korsakoff syndrome (WKS), is invariably a severe, selective amnesia against a background of relatively preserved general intellectual functions in a consecutive clinical sample. An analysis of the neuropsychological profiles of nine cases with a recorded history of WKS was undertaken. All cases were adult males (ages 32 to 70) with a long history of alcohol use disorder. Eight cases were chosen retrospectively on a consecutive basis from patient referrals. One additional case was recruited prospectively. Conventional understanding and some current opinion of Korsakoff’s syndrome predicts anterograde memory to be consistently more impaired than other cognitive abilities, but this was not found in this case series. The Mean Wechsler Delayed Memory Index was not significantly different from the Wechsler Full-Scale IQ (FSIQ), p = 0.130. Regression of Delayed Memory on FSIQ produced a non-significant intercept, p = 0.213. The ‘hallmark’ criterion of anterograde memory score at least 20 points less than intelligence score was observed in four of eight cases with available data, equating to a ‘sensitivity’ of 50%. Three of eight cases with available data had an FSIQ less than the memory score. Contrary to a common view, general intellectual function was not consistently preserved in Korsakoff’s syndrome relative to memory function. This study illustrates one of the specific merits of case series, namely, to critique an established view. Clinicians and researchers should expand their diagnostic criteria for Korsakoff’s syndrome to include more variable cognitive phenotypes, including a potentially reversible dementia-like impairment of variable severity, and focus on potential treatment opportunities. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
11 pages, 272 KiB  
Article
Self-Reported Pain and Pain Observations in People with Korsakoff’s Syndrome: A Pilot Study
by Erik Oudman, Thom van der Stadt, Janice R. Bidesie, Jan W. Wijnia and Albert Postma
J. Clin. Med. 2023, 12(14), 4681; https://doi.org/10.3390/jcm12144681 - 14 Jul 2023
Viewed by 1040
Abstract
Korsakoff’s syndrome (KS) is a chronic neuropsychiatric disorder. The large majority of people with KS experience multiple comorbid health problems, including cardiovascular disease, malignancy, and diabetes mellitus. To our knowledge pain has not been investigated in this population. The aim of this study [...] Read more.
Korsakoff’s syndrome (KS) is a chronic neuropsychiatric disorder. The large majority of people with KS experience multiple comorbid health problems, including cardiovascular disease, malignancy, and diabetes mellitus. To our knowledge pain has not been investigated in this population. The aim of this study was to investigate self-reported pain as well as pain behavior observations reported by nursing staff. In total, 38 people diagnosed with KS residing in a long-term care facility for KS participated in this research. The Visual Analogue Scale (VAS), Pain Assessment in Impaired Cognition (PAIC-15), Rotterdam Elderly Pain Observation Scale (REPOS), and the McGill Pain Questionnaire–Dutch Language Version (MPQ-DLV) were used to index self-rated and observational pain in KS. People with KS reported significantly lower pain levels than their healthcare professionals reported for them. The highest pain scores were found on the PAIC-15, specifically on the emotional expression scale. Of importance, the patient pain reports did not correlate with the healthcare pain reports. Moreover, there was a high correlation between neuropsychiatric symptoms and observational pain reports. Specifically, agitation and observational pain reports strongly correlated. In conclusion, people with KS report less pain than their healthcare professionals indicate for them. Moreover, there is a close relationship between neuropsychiatric symptoms and observation-reported pain in people with KS. Our results suggest that pain is possibly underreported by people with KS and should be taken into consideration in treating neuropsychiatric symptoms of KS as a possible underlying cause. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
11 pages, 1031 KiB  
Article
Antipsychotic Use and Mortality in Persons with Alcohol-Related Dementia or Wernicke–Korsakoff Syndrome: A Nationwide Register Study in Finland
by Anniina Palm, Tiina Talaslahti, Risto Vataja, Milena Ginters, Hannu Kautiainen, Henrik Elonheimo, Jaana Suvisaari, Nina Lindberg and Hannu Koponen
J. Clin. Med. 2023, 12(13), 4263; https://doi.org/10.3390/jcm12134263 - 25 Jun 2023
Viewed by 1456
Abstract
Background: Research on the use of psychotropic drugs in people with alcohol-related neurocognitive disorders is virtually nonexistent. We examined the prevalence of antipsychotic drug use and its effect on mortality among patients with Wernicke–Korsakoff syndrome (WKS) or alcohol-related dementia (ARD). Methods: In this [...] Read more.
Background: Research on the use of psychotropic drugs in people with alcohol-related neurocognitive disorders is virtually nonexistent. We examined the prevalence of antipsychotic drug use and its effect on mortality among patients with Wernicke–Korsakoff syndrome (WKS) or alcohol-related dementia (ARD). Methods: In this nationwide register study, we collected data on the medication use and mortality of all persons aged ≥40 diagnosed with WKS (n = 1149) or ARD (n = 2432) between 1998 and 2015 in Finland. We calculated the prevalence of antipsychotic use within one year of diagnosis and the adjusted cumulative mortality of antipsychotic users versus non-users in relation to the age-, sex-, and calendar year-matched general population. Results: Of the WKS and ARD patients, 35.9% and 38.5%, respectively, purchased one or more antipsychotic drugs in the year following diagnosis. The adjusted cumulative mortality of the antipsychotic users was significantly lower than that of non-users in both the WKS and ARD groups, where the adjusted hazard ratios (95% CI) were 0.85 (0.72–0.99) and 0.73 (0.65–0.81), respectively. WKS and ARD patients using antipsychotics were less likely to die of alcohol-related causes than antipsychotic non-users, but the difference was significant only in the ARD group. Conclusions: This population-based study shows that antipsychotic use is common in patients with WKS or ARD. In contrast to other dementia studies, our results indicate that the mortality of antipsychotic users is significantly lower than that of non-users. The lower mortality could be explained by decreased alcohol use and better healthcare coverage in antipsychotic users. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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9 pages, 600 KiB  
Article
Impaired Global Precedence Effect in Severe Alcohol Use Disorder and Korsakoff’s Syndrome: A Pilot Exploration through a Global/Local Visual Paradigm
by Anne Lise Pitel, Alice Laniepce, Céline Boudehent and Nicolas Poirel
J. Clin. Med. 2023, 12(11), 3655; https://doi.org/10.3390/jcm12113655 - 25 May 2023
Viewed by 950
Abstract
In healthy populations, visual abilities are characterized by a faster and more efficient processing of global features in a stimulus compared to local ones. This phenomenon is known as the global precedence effect (GPE), which is demonstrated by (1) a global advantage, resulting [...] Read more.
In healthy populations, visual abilities are characterized by a faster and more efficient processing of global features in a stimulus compared to local ones. This phenomenon is known as the global precedence effect (GPE), which is demonstrated by (1) a global advantage, resulting in faster response times for global features than local features and (2) interference from global distractors during the identification of local targets, but not vice versa. This GPE is essential for adapting visual processing in everyday life (e.g., extracting useful information from complex scenes). We investigated how the GPE is affected in patients with Korsakoff’s syndrome (KS) compared to patients with severe alcohol use disorder (sAUD). Three groups (including healthy controls, patients with KS and patients with sAUD) completed a global/local visual task in which predefined targets appeared at the global or local level during either congruent or incongruent (i.e., interference) situations. The results showed that healthy controls (N = 41) presented a classical GPE, while patients with sAUD (N = 16) presented neither a global advantage nor global interference effects. Patients with KS (N = 7) presented no global advantage and an inversion of the interference effect, characterized by strong interference from local information during global processing. The absence of the GPE in sAUD and the interference from local information in KS have implications in daily-life situations, providing preliminary data for a better understanding of how these patients perceive their visual world. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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22 pages, 1796 KiB  
Article
Eye Movements as Proxy for Visual Working Memory Usage: Increased Reliance on the External World in Korsakoff Syndrome
by Sanne Böing, Antonia F. Ten Brink, Alex J. Hoogerbrugge, Erik Oudman, Albert Postma, Tanja C. W. Nijboer and Stefan Van der Stigchel
J. Clin. Med. 2023, 12(11), 3630; https://doi.org/10.3390/jcm12113630 - 23 May 2023
Cited by 1 | Viewed by 1466
Abstract
In the assessment of visual working memory, estimating the maximum capacity is currently the gold standard. However, traditional tasks disregard that information generally remains available in the external world. Only when to-be-used information is not readily accessible, memory is taxed. Otherwise, people sample [...] Read more.
In the assessment of visual working memory, estimating the maximum capacity is currently the gold standard. However, traditional tasks disregard that information generally remains available in the external world. Only when to-be-used information is not readily accessible, memory is taxed. Otherwise, people sample information from the environment as a form of cognitive offloading. To investigate how memory deficits impact the trade-off between sampling externally or storing internally, we compared gaze behaviour of individuals with Korsakoff amnesia (n = 24, age range 47–74 years) and healthy controls (n = 27, age range 40–81 years) on a copy task that provoked different strategies by having information freely accessible (facilitating sampling) or introducing a gaze-contingent waiting time (provoking storing). Indeed, patients sampled more often and longer, compared to controls. When sampling became time-consuming, controls reduced sampling and memorised more. Patients also showed reduced and longer sampling in this condition, suggesting an attempt at memorisation. Importantly, however, patients sampled disproportionately more often than controls, whilst accuracy dropped. This finding suggests that amnesia patients sample frequently and do not fully compensate for increased sampling costs by memorising more at once. In other words, Korsakoff amnesia resulted in a heavy reliance on the world as ‘external memory’. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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10 pages, 994 KiB  
Article
Korsakoff’s Syndrome and Alzheimer’s Disease—Commonalities and Specificities of Volumetric Brain Alterations within Papez Circuit
by Shailendra Segobin, Melanie Ambler, Alice Laniepce, Hervé Platel, Gael Chételat, Mathilde Groussard and Anne-Lise Pitel
J. Clin. Med. 2023, 12(9), 3147; https://doi.org/10.3390/jcm12093147 - 27 Apr 2023
Cited by 1 | Viewed by 1762
Abstract
Background: Alzheimer’s disease (AD) and Korsakoff’s syndrome (KS) are two major neurocognitive disorders characterized by amnesia but AD is degenerative while KS is not. The objective is to compare regional volume deficits within the Papez circuit in AD and KS, considering AD [...] Read more.
Background: Alzheimer’s disease (AD) and Korsakoff’s syndrome (KS) are two major neurocognitive disorders characterized by amnesia but AD is degenerative while KS is not. The objective is to compare regional volume deficits within the Papez circuit in AD and KS, considering AD progression. Methods: 18 KS patients, 40 AD patients (20 with Moderate AD (MAD) matched on global cognitive deficits with KS patients and 20 with Severe AD (SAD)), and 70 healthy controls underwent structural MRI. Volumes of the hippocampi, thalami, cingulate gyri, mammillary bodies (MB) and mammillothalamic tracts (MTT) were extracted. Results: For the cingulate gyri, and anterior thalamic nuclei, all patient groups were affected compared to controls but did not differ between each other. Smaller volumes were observed in all patient groups compared to controls in the mediodorsal thalamic nuclei and MB, but these regions were more severely damaged in KS than AD. MTT volumes were damaged in KS only. Hippocampi were affected in all patient groups but more severely in the SAD than in the KS and MAD. Conclusions: There are commonalities in the pattern of volume deficits in KS and AD within the Papez circuit with the anterior thalamic nuclei, cingulate cortex and hippocampus (in MAD only) being damaged to the same extent. The specificity of KS relies on the alteration of the MTT and the severity of the MB shrinkage. Further comparative studies including other imaging modalities and a neuropsychological assessment are required. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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14 pages, 516 KiB  
Article
Impaired Awareness in People with Severe Alcohol-Related Cognitive Deficits Including Korskoff’s Syndrome: A Network Analysis
by Hester Fidder, Ruth B. Veenhuizen, Ineke J. Gerridzen, Wessel N. van Wieringen, Martin Smalbrugge, Cees M. P. M. Hertogh and Anouk M. van Loon
J. Clin. Med. 2023, 12(9), 3139; https://doi.org/10.3390/jcm12093139 - 26 Apr 2023
Viewed by 1244
Abstract
Background: Impaired awareness of one’s own functioning is highly common in people with Korsakoff’s syndrome (KS). However, it is currently unclear how awareness relates to impairments in daily functioning and quality of life (QoL). Methods: We assessed how impaired awareness relates to cognitive, [...] Read more.
Background: Impaired awareness of one’s own functioning is highly common in people with Korsakoff’s syndrome (KS). However, it is currently unclear how awareness relates to impairments in daily functioning and quality of life (QoL). Methods: We assessed how impaired awareness relates to cognitive, behavioral, physical, and social functioning and QoL by applying a network analysis. We used cross-sectional data from 215 patients with KS or other severe alcohol-related cognitive deficits living in Dutch long-term care facilities (LTCFs). Results: Apathy has the most central position in the network. Higher apathy scores relate positively to reduced cognition and to a greater decline in activities of daily living and negatively to social participation and the use of antipsychotic drugs. Impaired awareness is also a central node. It is positively related to a higher perceived QoL, reduced cognition and apathy, and negatively to social participation and length of stay in the LTCF. Mediated through apathy and social participation, impaired awareness is indirectly related to other neuropsychiatric symptoms. Conclusions: Impaired awareness is closely related to other domains of daily functioning and QoL of people with KS or other severe alcohol-related cognitive deficits living in LTCFs. Apathy plays a central role. Network analysis offers interesting insights to evaluate the interconnection of different symptoms and impairments in brain disorders such as KS. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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11 pages, 425 KiB  
Article
Prevalences and Indications of Psychotropic Drug Prescriptions in Nursing Home Residents with Korsakoff Syndrome
by Ineke J. Gerridzen, Els Doejaaren, Ruth B. Veenhuizen, Cees M. P. M. Hertogh and Karlijn J. Joling
J. Clin. Med. 2023, 12(9), 3133; https://doi.org/10.3390/jcm12093133 - 26 Apr 2023
Cited by 1 | Viewed by 1313
Abstract
Psychotropic drugs (PD) are often prescribed to nursing home residents with Korsakoff syndrome (KS). It is unknown whether these drugs are prescribed correctly or whether they are prescribed off-label, for example, to treat behavioral symptoms. To get more insight into PD prescriptions, a [...] Read more.
Psychotropic drugs (PD) are often prescribed to nursing home residents with Korsakoff syndrome (KS). It is unknown whether these drugs are prescribed correctly or whether they are prescribed off-label, for example, to treat behavioral symptoms. To get more insight into PD prescriptions, a descriptive study was performed. The type, category and indications of PD prescriptions of 285 participants were analyzed using medication charts and questionnaires. Behavioral symptoms were investigated with the Neuropsychiatric Inventory-Questionnaire. The results showed that atypical antipsychotics (57.1%) were prescribed more frequently than typical antipsychotics (49.3%). Of the antidepressants, selective serotonin/norepinephrine reuptake inhibitors (63.1%) were most frequently prescribed, followed by tricyclic antidepressants (23.4%). Of the benzodiazepines, anxiolytics (85.7%) were more prescribed than hypnotics (24.5%). Besides psychiatric disorders, PD were also prescribed to treat behavioral symptoms varying from 29.9% (antipsycho-tics) to 26.3% (benzodiazepines) and 9.3% (antidepressants). Furthermore, prescriptions were high if behavioral symptoms were present. To conclude, PD are often prescribed to residents with KS for an unapproved indication, namely behavioral symptoms. Additional research is needed to obtain further insight into the current prescribing culture and the effectiveness of PD. The insights thus obtained may, ultimately, contribute to the appropriate prescription of PD for people with KS. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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15 pages, 3131 KiB  
Article
Coordination and Cognition in Pure Nutritional Wernicke’s Encephalopathy with Cerebellar Degeneration after COVID-19 Infection: A Unique Case Report
by Nicolaas J. M. Arts, Maud E. G. van Dorst, Sandra H. Vos and Roy P. C. Kessels
J. Clin. Med. 2023, 12(7), 2511; https://doi.org/10.3390/jcm12072511 - 27 Mar 2023
Viewed by 2471
Abstract
Background: Alcoholic cerebellar degeneration is a restricted form of cerebellar degeneration, clinically leading to an ataxia of stance and gait and occurring in the context of alcohol misuse in combination with malnutrition and thiamine depletion. However, a similar degeneration may also develop after [...] Read more.
Background: Alcoholic cerebellar degeneration is a restricted form of cerebellar degeneration, clinically leading to an ataxia of stance and gait and occurring in the context of alcohol misuse in combination with malnutrition and thiamine depletion. However, a similar degeneration may also develop after non-alcoholic malnutrition, but evidence for a lasting ataxia of stance and gait and lasting abnormalities in the cerebellum is lacking in the few patients described with purely nutritional cerebellar degeneration (NCD). Methods: We present a case of a 46-year-old woman who developed NCD and Wernicke’s encephalopathy (WE) due to COVID-19 and protracted vomiting, resulting in thiamine depletion. We present her clinical course over the first 6 months after the diagnosis of NCD and WE, with thorough neuropsychological and neurological examinations, standardized clinical observations, laboratory investigations, and repeated MRIs. Results: We found a persistent ataxia of stance and gait and evidence for an irreversible restricted cerebellar degeneration. However, the initial cognitive impairments resolved. Conclusions: Our study shows that NCD without involvement of alcohol neurotoxicity and with a characteristic ataxia of stance and gait exists and may be irreversible. We did not find any evidence for lasting cognitive abnormalities or a cerebellar cognitive-affective syndrome (CCAS) in this patient. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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17 pages, 1689 KiB  
Article
Distinct Sleep Alterations in Alcohol Use Disorder Patients with and without Korsakoff’s Syndrome: Relationship with Episodic Memory
by Alice Laniepce, Shailendra Segobin, Claire André, Françoise Bertran, Céline Boudehent, Najlaa Lahbairi, Angéline Maillard, Alison Mary, Laurent Urso, François Vabret, Nicolas Cabé, Anne-Lise Pitel and Géraldine Rauchs
J. Clin. Med. 2023, 12(6), 2440; https://doi.org/10.3390/jcm12062440 - 22 Mar 2023
Viewed by 1613
Abstract
Alcohol Use Disorder (AUD) results in sleep disturbances that may have deleterious impacts on cognition, especially on memory. However, little is known about the sleep architecture in patients with Korsakoff’s syndrome (KS). This study aims at characterizing sleep disturbances in KS compared to [...] Read more.
Alcohol Use Disorder (AUD) results in sleep disturbances that may have deleterious impacts on cognition, especially on memory. However, little is known about the sleep architecture in patients with Korsakoff’s syndrome (KS). This study aims at characterizing sleep disturbances in KS compared to AUD without KS and at specifying the relationships with cognitive impairments. Twenty-nine AUD patients (22 without KS and 7 with KS) and 15 healthy controls underwent a neuropsychological assessment and a polysomnography. The severity of sleep-disordered breathing and sleep fragmentation was similar in AUD and KS patients compared to controls. Sleep architecture differed between both patient groups: the proportion of slow-wave sleep was reduced in AUD patients only, while a lower proportion of rapid-eye movement (REM) sleep was specifically observed in KS patients. The proportion of REM sleep correlated with the severity of episodic memory deficits when AUD and KS were examined together. These data provide evidence for both similarities and specificities regarding sleep alterations in AUD patients with and without KS. They also indicate that altered sleep architecture may contribute to the pathophysiology of alcohol-related memory disorders. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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10 pages, 288 KiB  
Article
Factors Associated with ADL Dependence in Nursing Home Residents with Korsakoff’s Syndrome and Other Alcohol-Related Disorders: An Explorative Cross-Sectional Study
by Eline S. Böhner, Bea Spek, Karlijn J. Joling, Yvonne Zwaagstra and Ineke J. Gerridzen
J. Clin. Med. 2023, 12(6), 2181; https://doi.org/10.3390/jcm12062181 - 11 Mar 2023
Cited by 3 | Viewed by 1667
Abstract
Difficulties in performing activities of daily living (ADL) are common in patients with Korsakoff‘s syndrome (KS). The aim of this study was to identify factors associated with ADL dependence in nursing home residents with KS. This exploratory, cross-sectional study included 281 residents with [...] Read more.
Difficulties in performing activities of daily living (ADL) are common in patients with Korsakoff‘s syndrome (KS). The aim of this study was to identify factors associated with ADL dependence in nursing home residents with KS. This exploratory, cross-sectional study included 281 residents with KS from 9 specialized nursing homes in the Netherlands. We examined demographic, cognitive, somatic, and (neuro)psychiatric characteristics. ADL dependence was assessed with the Inter-RAI ADL Hierarchy Scale. Multivariable logistic regression analyses were used to identify factors associated with ADL dependence. Cognitive impairment (odds ratio [OR] = 7.46; 95% confidence interval [CI] = 2.10–30.5), female gender (OR = 3.23; CI, 1.21–8.78), staying in a nursing home for ≥5 years (OR = 3.12; CI, 1.24–8.33), and impaired awareness (OR = 4.25; CI, 1.56–12.32) were significantly associated with higher ADL dependence. Chronic obstructive pulmonary disease (COPD) was significantly associated with lower ADL dependence (OR = 0.31; CI, 0.01–0.84). The model explained 32% of the variance. The results suggest that when choosing interventions aimed at improving ADL functioning, special attention should be paid to residents living more than five years in the nursing home, with a female gender, with more severe cognitive impairments, and/or with COPD. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
11 pages, 521 KiB  
Article
“What Did I Tell This Sad Person?”: Memory for Emotional Destinations in Korsakoff’s Syndrome
by Mohamad El Haj, André Ndobo, Ahmed A. Moustafa and Philippe Allain
J. Clin. Med. 2023, 12(5), 1919; https://doi.org/10.3390/jcm12051919 - 28 Feb 2023
Cited by 4 | Viewed by 1238
Abstract
We investigated destination memory, defined as the ability to remember to whom a piece of information was previously transmitted, for emotional destinations (i.e., a happy or sad person) in Korsakoff’s syndrome (KS). We asked patients with KS and control participants to tell facts [...] Read more.
We investigated destination memory, defined as the ability to remember to whom a piece of information was previously transmitted, for emotional destinations (i.e., a happy or sad person) in Korsakoff’s syndrome (KS). We asked patients with KS and control participants to tell facts to neutral, positive, or negative faces. On a subsequent recognition task, participants had to decide to whom they told each fact. Compared with control participants, patients with KS demonstrated lower recognition of neutral, emotionally positive, and emotionally negative destinations. Patients with KS demonstrated lower recognition of emotionally negative than for emotionally positive or neutral destinations, but there were no significant differences between recognition of neutral and emotionally positive destinations. Our study demonstrates a compromised ability to process negative destinations in KS. Our study highlights the relationship between memory decline and impaired emotional processing in KS. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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12 pages, 2084 KiB  
Article
The Use of an Errorless Learning Application to Support Re-Learning of (Instrumental) Activities for People Living with Korsakoff Syndrome
by Roeline Biemond, Erik Oudman and Albert Postma
J. Clin. Med. 2022, 11(23), 6947; https://doi.org/10.3390/jcm11236947 - 25 Nov 2022
Cited by 2 | Viewed by 1533
Abstract
Korsakoff syndrome (KS) is a severe neuropsychiatric syndrome derived from acute thiamine deficiency and concomitant alcohol use disorders. KS patients need lifelong assistance because of the severity of their cognitive problems. In clinical practice and research, errorless learning has proven to be an [...] Read more.
Korsakoff syndrome (KS) is a severe neuropsychiatric syndrome derived from acute thiamine deficiency and concomitant alcohol use disorders. KS patients need lifelong assistance because of the severity of their cognitive problems. In clinical practice and research, errorless learning has proven to be an effective cognitive rehabilitation method for patients with KS. Our study focused on optimizing errorless learning by introducing new software technology to support the training process of errorless learning. Although the benefits of errorless learning for patients with Korsakoff’s syndrome have been thoroughly investigated, it is currently unclear whether new technology could contribute to better learning and maintenance of everyday tasks. Therefore, an errorless learning application was built. This device is a web application and can be used on a tablet, laptop, or smartphone. The application allows clinicians and researchers to insert pictures, videoclips, timers, and audio fragments in the different steps of an errorless learning training plan. This way, the different steps are visible and easy to follow for patients. Moreover, it ensures as a learning method that the training is executed exactly the same way for each and every training. The aim of this study was twofold: to examine whether the use of the errorless learning application is effective, and whether it leads to better results than a regular errorless learning of everyday activities. In total, 13 patients with KS were trained in instrumental activities of daily living by means of the application, and 10 patients were trained with traditional instructions. Results showed an equal improvement for both training methods. Importantly, the technology group could better remember the training when probed at a later moment than the traditional errorless learning group. These results are promising for further development of novel technology to support errorless learning applications in clinical practice. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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Review

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13 pages, 292 KiB  
Review
Observations on the Clinical Features of the Wernicke–Korsakoff Syndrome
by Michael D. Kopelman
J. Clin. Med. 2023, 12(19), 6310; https://doi.org/10.3390/jcm12196310 - 30 Sep 2023
Viewed by 1342
Abstract
This paper begins with a short case report of florid, spontaneous confabulation in a 61-year-old man with an alcohol-induced Wernicke-Korsakoff syndrome. His confabulation extended across episodic and personal semantic memory, as well as orientation in time and place, as measured on Dalla Barba’s [...] Read more.
This paper begins with a short case report of florid, spontaneous confabulation in a 61-year-old man with an alcohol-induced Wernicke-Korsakoff syndrome. His confabulation extended across episodic and personal semantic memory, as well as orientation in time and place, as measured on Dalla Barba’s Confabulation Battery. Five other brief case summaries will then be presented, followed by a summary of the clinical, neurological, and background neuropsychological findings in three earlier series of Korsakoff patients. These observations will be considered in light of Wijnia’s recent and my own, earlier reviews of the Korsakoff syndrome. Taken together, they indicate the need for a multi-faceted approach (clinical, neurological, neuropsychological, and neuroimaging) to the assessment and diagnosis of the disorder. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
19 pages, 721 KiB  
Review
Neuropsychiatric and Neuropsychological Aspects of Alcohol-Related Cognitive Disorders: An In-Depth Review of Wernicke’s Encephalopathy and Korsakoff’s Syndrome
by Lucian Eva, Felix-Mircea Brehar, Ioan-Alexandru Florian, Razvan-Adrian Covache-Busuioc, Horia Petre Costin, David-Ioan Dumitrascu, Bogdan-Gabriel Bratu, Luca-Andrei Glavan and Alexandru Vlad Ciurea
J. Clin. Med. 2023, 12(18), 6101; https://doi.org/10.3390/jcm12186101 - 21 Sep 2023
Viewed by 2493
Abstract
Alcohol-related cognitive disorders have long been an area of study, yet they continue to pose challenges in the diagnosis, treatment, and understanding of underlying neuropsychiatric mechanisms. The present article offers a comprehensive review of Wernicke’s Encephalopathy and Korsakoff’s Syndrome, two conditions often seen [...] Read more.
Alcohol-related cognitive disorders have long been an area of study, yet they continue to pose challenges in the diagnosis, treatment, and understanding of underlying neuropsychiatric mechanisms. The present article offers a comprehensive review of Wernicke’s Encephalopathy and Korsakoff’s Syndrome, two conditions often seen on a continuum of alcohol-related brain damage. Drawing on current medical literature, neuroimaging studies, and clinical case reports, we explore the neuropsychiatric and neuropsychological profiles, symptomatology, and differential diagnoses of these disorders. We delve into the biochemical pathways implicated in the development of WE and KS, notably thiamine deficiency and its impact on neurotransmitter systems and neural networks. The article also addresses the challenges in early diagnosis, often complicated by non-specific symptoms and co-occurring psychiatric conditions. Furthermore, we review the current state of treatment protocols, including pharmacological and non-pharmacological interventions. Finally, the article highlights gaps in current knowledge and suggests directions for future research to improve diagnosis, treatment, and patient outcomes. Understanding the nuanced interplay between the neuropsychiatric and neuropsychological aspects of WE and KS is crucial for both clinicians and researchers alike, in order to provide effective treatment and to advance our understanding of these complex conditions. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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13 pages, 918 KiB  
Review
A Clinician’s View of Wernicke-Korsakoff Syndrome
by Jan W. Wijnia
J. Clin. Med. 2022, 11(22), 6755; https://doi.org/10.3390/jcm11226755 - 15 Nov 2022
Cited by 15 | Viewed by 9110
Abstract
The purpose of this article is to improve recognition and treatment of Wernicke-Korsakoff syndrome. It is well known that Korsakoff syndrome is a chronic amnesia resulting from unrecognized or undertreated Wernicke encephalopathy and is caused by thiamine (vitamin B1) deficiency. The clinical presentation [...] Read more.
The purpose of this article is to improve recognition and treatment of Wernicke-Korsakoff syndrome. It is well known that Korsakoff syndrome is a chronic amnesia resulting from unrecognized or undertreated Wernicke encephalopathy and is caused by thiamine (vitamin B1) deficiency. The clinical presentation of thiamine deficiency includes loss of appetite, dizziness, tachycardia, and urinary bladder retention. These symptoms can be attributed to anticholinergic autonomic dysfunction, as well as confusion or delirium, which is part of the classic triad of Wernicke encephalopathy. Severe concomitant infections including sepsis of unknown origin are common during the Wernicke phase. These infections can be prodromal signs of severe thiamine deficiency, as has been shown in select case descriptions which present infections and lactic acidosis. The clinical symptoms of Wernicke delirium commonly arise within a few days before or during hospitalization and may occur as part of a refeeding syndrome. Wernicke encephalopathy is mostly related to alcohol addiction, but can also occur in other conditions, such as bariatric surgery, hyperemesis gravidarum, and anorexia nervosa. Alcohol related Wernicke encephalopathy may be identified by the presence of a delirium in malnourished alcoholic patients who have trouble walking. The onset of non-alcohol-related Wernicke encephalopathy is often characterized by vomiting, weight loss, and symptoms such as visual complaints due to optic neuropathy in thiamine deficiency. Regarding thiamine therapy, patients with hypomagnesemia may fail to respond to thiamine. This may especially be the case in the context of alcohol withdrawal or in adverse side effects of proton pump inhibitors combined with diuretics. Clinician awareness of the clinical significance of Wernicke delirium, urinary bladder retention, comorbid infections, refeeding syndrome, and hypomagnesemia may contribute to the recognition and treatment of the Wernicke-Korsakoff syndrome. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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2 pages, 195 KiB  
Comment
Comment on Wijnia, J.W. A Clinician’s View of Wernicke-Korsakoff Syndrome. J. Clin. Med. 2022, 11, 6755
by Michele Manigrasso, Nunzio Velotti, Giovanni Domenico De Palma and Mario Musella
J. Clin. Med. 2023, 12(19), 6393; https://doi.org/10.3390/jcm12196393 - 7 Oct 2023
Cited by 1 | Viewed by 515
Abstract
We have read with great interest the article by Wijnia [...] Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
8 pages, 228 KiB  
Perspective
Music Therapy and Korsakoff’s Syndrome: The State of the Art
by Monique van Bruggen-Rufi and Gerjanne van der Stouw
J. Clin. Med. 2023, 12(14), 4609; https://doi.org/10.3390/jcm12144609 - 11 Jul 2023
Cited by 1 | Viewed by 1213
Abstract
In this perspective article, the authors give insight into the beneficial effects and the current developments in music therapy for patients with Korsakoff’s syndrome (KS) in the Netherlands. Music may be the key to distracting patients from negative moods, to help them express [...] Read more.
In this perspective article, the authors give insight into the beneficial effects and the current developments in music therapy for patients with Korsakoff’s syndrome (KS) in the Netherlands. Music may be the key to distracting patients from negative moods, to help them express emotions and to teach them new skills on physical, psychosocial and cognitive levels. This may lead to improving the quality of life of patients with KS. Emphasis is placed on the personal experience of the authors and on the future directions in the field. Their experience, as well as the experience of music therapy colleagues working in the field with the same target population (joined together in the Music Therapy Korsakoff Expertise Group), is situated in the context of existing literature and showcases current developments in the specific field of music therapy and KS. Since literature on this specific topic is limited, the authors allowed themselves to delve into somewhat older but still leading and representative literature. There is still little knowledge on how music therapy may contribute to reducing the impairments patients with KS suffer and to improving their quality of life in general. Using the Empathic Directive Approach (EDA) as the starting point, the authors elaborate on different potential approaches and interventions. With this article, the authors aim to gain more insight into the potential role of the music therapist by highlighting music–therapeutic micro-interventions and to provide recommendations for future directions on how to integrate music therapy in the treatment of patients with Korsakoff’s syndrome. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
10 pages, 1268 KiB  
Case Report
Young Nonalcoholic Wernicke Encephalopathy Patient Achieves Remission Following Prolonged Thiamine Treatment and Cognitive Rehabilitation
by Erik Oudman, Jan W. Wijnia, Janice Bidesie, Zyneb Al-Hassaan, Sascha Laenen and Amy V. Jong-Tjien-Fa
J. Clin. Med. 2023, 12(8), 2901; https://doi.org/10.3390/jcm12082901 - 17 Apr 2023
Cited by 4 | Viewed by 2573
Abstract
Wernicke encephalopathy (WE), a neurological emergency commonly associated with alcohol use disorder, results from a severe deficiency of vitamin B1. If left untreated, patients either succumb to the illness or develop chronic Korsakoff’s syndrome (KS). Recently, an increasing number of nonalcoholic WE case [...] Read more.
Wernicke encephalopathy (WE), a neurological emergency commonly associated with alcohol use disorder, results from a severe deficiency of vitamin B1. If left untreated, patients either succumb to the illness or develop chronic Korsakoff’s syndrome (KS). Recently, an increasing number of nonalcoholic WE case studies have been published, highlighting a lack of understanding of malnutrition-related disorders among high-functioning patients. We present the case of a 26 year old female who developed life-threatening WE after COVID-19-complicated obesity surgery. She experienced the full triad of WE symptoms, including eye-movement disorders, delirium, and ataxia, and suffered for over 70 days before receiving her initial WE diagnosis. Late treatment resulted in progression of WE symptoms. Despite the severity, the patient achieved remission of some of the symptoms in the post-acute phase due to prolonged parenteral thiamine injections and intensive specialized rehabilitation designed for young traumatic brain injury (TBI) patients. The rehabilitation resulted in gradual remission of amnesia symptomatology, mainly increasing her autonomy. The late recognition of this case highlights the importance of early diagnosis and prompt, targeted intervention in the management of nonalcoholic WE, as well as underscores the potential for positive outcomes after delayed treatment through intensive cognitive rehabilitation in specialized treatment centers. Full article
(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)
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